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Ultrasonographic nerve enlargement of the median and ulnar nerves and the cervical nerve roots in patients with demyelinating Charcot–Marie–Tooth disease: distinction from patients with chronic inflammatory demyelinating polyneuropathy

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Abstract

Demyelinating Charcot–Marie–Tooth disease (CMT) and chronic inflammatory demyelinating polyneuropathy (CIDP) are both demyelinating polyneuropathies. The differences in nerve enlargement degree and pattern at multiple evaluation sites/levels are not well known. We investigated the differences in nerve enlargement degree and the distribution pattern of nerve enlargement in patients with demyelinating CMT and CIDP, and verified the appropriate combination of sites/levels to differentiate between these diseases. Ten patients (aged 23–84 years, three females) with demyelinating CMT and 16 patients (aged 30–85 years, five females) with CIDP were evaluated in this study. The nerve sizes were measured at 24 predetermined sites/levels from the median and ulnar nerves and the cervical nerve roots (CNR) using ultrasonography. The evaluation sites/levels were classified into three regions: distal, intermediate and cervical. The number of sites/levels that exhibited nerve enlargement (enlargement site number, ESN) in each region was determined from the 24 sites/levels and from the selected eight screening sites/levels, respectively. The cross-sectional areas of the peripheral nerves were markedly larger at all evaluation sites in patients with demyelinating CMT than in patients with CIDP (p < 0.01). However, the nerve sizes of CNR were not significantly different between patients with either disease. When we evaluated ESN of four selected sites for screening from the intermediate region, the sensitivity and specificity to distinguish between demyelinating CMT and CIDP were 0.90 and 0.94, respectively, with the cut-off value set at four. Nerve ultrasonography is useful to detect nerve enlargement and can clarify morphological differences in nerves between patients with demyelinating CMT and CIDP.

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Acknowledgments

We would like to sincerely thank Dr. Takemori Yamawaki for his insightful comments. This work was supported in part by a Grant-in-Aid from the Neuroimmunological Disease Research Committee of the Ministry of Health, Labour and Welfare of Japan.

Conflicts of interest

The authors declare no conflicts of interest.

Ethical standard

This study was approved by the ethics committee of Hiroshima University Hospital and was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Authors and Affiliations

  1. Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan

    Takamichi Sugimoto, Kazuhide Ochi, Naohisa Hosomi, Tetsuya Takahashi, Hiroki Ueno, Takeshi Nakamura, Yoshito Nagano, Hirofumi Maruyama & Masayasu Matsumoto

  2. Hiroshima City General Rehabilitation Center, 1-39-1 Tomominami, Asaminami-ku, Hiroshima, 731-3168, Japan

    Tatsuo Kohriyama

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  1. Takamichi Sugimoto
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  2. Kazuhide Ochi
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  3. Naohisa Hosomi
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  10. Masayasu Matsumoto
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Correspondence to Takamichi Sugimoto.

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Sugimoto, T., Ochi, K., Hosomi, N. et al. Ultrasonographic nerve enlargement of the median and ulnar nerves and the cervical nerve roots in patients with demyelinating Charcot–Marie–Tooth disease: distinction from patients with chronic inflammatory demyelinating polyneuropathy. J Neurol 260, 2580–2587 (2013). https://doi.org/10.1007/s00415-013-7021-0

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  • DOI: https://doi.org/10.1007/s00415-013-7021-0

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